AB0663 Neurological involvement in primary sjÖgren syndrome

Abstract

Background Primary Sjögren Syndrome (pSS) is an autoimmune disorder characterized by a chronic lymphocytic and plasmacellular infiltration of exocrine glands and extraglandular features including both peripheral nervous system and central nervous system involvement. However, the diagnosis of pSS with neurologic involvement is sometimes difficult and specially central nervous system manifestations have been rarely described. Objectives To perform an observational retrospective cross-sectional case–control study to evaluate prevalence, clinical patterns and outcomes of neurological involvement in a cohort of pSS patients followed up in a single centre. Methods A total of 91 pSS patients (88 females, 3 male) with a mean age 47,6 years, diagnosed according to the 2002 criteria proposed by the American–European Consensus Group, were evaluated for neurological involvement after exclusion of secondary causes. Demographic, clinical, immunological data were compared between patients with and without neurological involvement. Neuroimaging data and peripheral nerve conduction study with quantitative sensory testing were also analysed. Results Neurological involvement was detected in 24 (26, 4%) patients (all females). The mean age at disease onset and neurological onset were 41,8 and 47,9 years, respectively. Neurologic involvement frequently preceded the diagnosis of pSS in 10 (42%) patients. Fifty (n=12) patients had peripheral system involvement (PNS), 46% (n=11) patients had CNS disorders and 4% (n=1) patients had both PNS and CNS involvement. In patients with PNS involvement, pure sensory neuropathy (small fiber neuropathy confirmed by quantitative sensory testing and sural neuropathy) occurred most frequently (n=5), followed by cranial nerve involvement affecting trigeminal, facial, or trochlear nerves (n=4). Multiple mononeuropathy (n=1), sensorimotor polyneuropathy (n=1), autonomic neuropathy (n=1) and myasthenia gravis (n=1), were also observed. In patients with CNS disorders, headache with MRI abnormalities compatible with inflammatory disease (n=3) occurred most frequently. Spinal cord involvement (n=2), seizures (n=2), motor and sensory deficit (n=2), movement disorders (n=2), aseptic meningitis (n=1) were the other manifestations observed. Cognitive dysfunction was observed in 3 of these patients. Headache was reported in nine patients other than the three mentioned above. The Raynaud’s phenomenon was more common in these patients (p=0, 04).The frequency of constitutional symptoms (such as fever and fatigue) and lung involvement was significantly higher (p<0,05) in pSS with neurological involvement than in pSS without neurological involvement and the articular symptoms were significantly less frequent (p<0,05) in pSS with neurological involvement. There was no statisticalsignificance in other factors parameters between the two groups. The neurologic outcome was good in 58% (n=14) patients. Conclusions Our results showed that neurological complications were present in 26, 4% of all patients with pSS. In other...

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…Transverse myelitis has also been described in patients with pSS [3,82,83], and in a small percentage it may be accompanied by brain MS-like lesions or by optic neuritis making, at times, the distinction from a primary demyelinating disease such as MS or NMO rather difficult [31,80]. The meninges can also be affected, alone or in the context of meningoencephalitis [84], while in some cases a subacute encephalopathy presenting with memory loss, cognitive dysfunction, visual disturbances, and reduced concentration and attention has been noted [80].…”

“…Symptoms such as seizures, headaches, psychiatric disturbances, and cognitive dysfunction have also been described but their true prevalence and association with the underlying syndrome is questionable [3,82]. Isolated cases of optic neuropathy, chorea, and motor neuron syndromes can also exist [82,83], but there does not appear to be any relationship or pathogenetic mechanism with the primary disease.…”

“…Peripheral neuropathies are well documented in pSS, even though their prevalence varies greatly among studies (ranging from 2 % to 60 %) [83,89,90]. Some studies have found that symmetric axonal sensory or sensorimotor polyneuropathy is the most common form of neuropathy (34.0 %), followed by cranial neuropathies (19.5 %), multiple mononeuropathies (8.5 %), ganglionopathies (<5 %), and CIDP (1.3 %) [20,82,90].…”

Bilateral Internuclear Ophthalmoplegia as a Presenting Manifestation of Primary Sjögren’s Syndrome

“…10 Among them, the most frequent are headache, movement disorders, seizures, myelopathy, aseptic meningitis, and cognitive dysfunction. 4,10,11 The pathological processes causing the CNS involvement include mononuclear lymphocytic infiltration in the meninges or parenchyma, microinfarcts, or microhaemorrhages in brain parenchyma.…”

Autoimmune Diseases Associated with Epilepsy

“…[62] Primer SS hastalarında epileptik nöbetler oldukça nadirdir (%3-7). [63,64] Epileptik nöbetler genellikle sicca semptomlaTablo 4. Sjögren Sendromu (SS) Amerika-Avrupa ortak tanı kriterleri 2002 [55] Primer SS 1. lı dural sinüs trombozu olguları ile diğer nedenlere bağlı dural sinüs trombozu olgularının karşılaştırıldığı bir çalış-mada, birinci grupta 36 olgudan sadece birinde (%3) epileptik nöbetler görülürken, diğer grupta bu oran %50 bulunmuştur.…”